Week 3: The Blood and Body defences: Too Much or Too Little Flashcards
What is polycythemia vera?
An excess of red blood cells in the circulation
What is essential thrombocytopenia?
Excess of Platelets
What is idiopathic myelofibrosis?
Too few RBC, too many platelets and WBC
What is primary polycthemia?
Genetic Problems with RBC - primary familial and congenital polycythaemia due to enhanced responsiveness to EPO. Most commonly due to Mutations in JAK 2
What is primary polycthemia?
Genetic Problems with RBC - primary familial and congenital polycythaemia due to enhanced responsiveness to EPO. Most commonly due to Mutations in JAK 2
What is secondary polycthaemia?
Due to conditions that promote RBC development.
1) Hypoxia
2) EPO secreting tumors
3) Neonatal Polycythaemia
What is relative polycythaemia?
RBCs normal but reduced plasma volume
What is JAK 2 gene?
Non receptor tyrosine kinase
How does the mutation effect JAK2?
The mutation takes place in the TK like domain which is responsible for the inhibitory role
The mutation, therefore, makes JAK2 constitutively active in the absence of ligand binding
What are the features of type 1 (Immediate) Hypersensitivity?
Immune reactant= IgE
Antigen = Soluble antigen
Effector Mechanism = Mast cell activation
Examples of hypersensitivity reaction: allergic rhinitis, asthma, anaphylaxis
Mast cell binds on the Fc component of IgE
What are the consequences of type 1 hypersensitivity?
What are the features of type 2 hypersensitivity?
what are the features of type 3 hs (immune complex disease)?
can effect renal glomerule
What are the features of type 4 hs (delayed)?
Why is there a delay in type 4 HS?
Takes time for Th1 cells to recognise antigens, release cytokines and and recruit other leukocytes
Example of primary immunodeficiency?
Chronic Granulomatous disease
Example of secondary immunodeficiency?
HIV/AIDS
What is chronic gramatulous disease?
A genetic disorder that affects how phagocytes kill bacteria, fungi and parasites
4 major forms due to mutation in the different proteins that comprise the phagocyte oxidase system
Recessive Sex-linked
What is HIV?
A retroviral infection leading to loss of CD4+ T cells
What is the impact of secondary immunodeficiency on T cell regulation?
By reducing the number of CD4+ T cells, there is also a reduction in the T cells that it differentiates into such as T reg cells, Th1, Th2 and Th17 and cytokines and TFs.
What is autoinflammation?
Prolonged, unwanted Innate Immunity
What is autoimmunity?
Loss/ failure of self/ non-self recognition
What is tolerance in immuntiy?
When T cells/B cells that can recognise self proteins are removed during development
What are 4 ways in which autoimmune diseases can occur by infection?
Molecular Mimicry
Protein Changes, Cryptic Antigens
Super Antigens
Bystander Activation
What therapeutic interventions can be used in autoimmune and autoinflammatory disease?
Cytokine therapies
Reducing Lymphocyte Activation - Interefering with signalling pathways, Blocking receptors and active induction of tolerance
Killing the cells causing disease
